[Acquired hemophilia as the initial manifestation of colorectal cancer's recurrence]. / Hemofilia adquirida como manifestación inicial de recidiva de adenocarcinoma colorrectal.

We report the case of an 86-year-old man presenting with a spontaneous hematoma in the left iliac muscle and previous diagnosis of colon cancer in 1998 (stage pT3N0M0) treated with transverse colectomy and considered in complete remission. After a complete study, it was possible to identify the presence of Factor VIII inhibitors antibodies that confirmed the presence of acquired hemophilia. During hospitalization the patient presented a lower gastrointestinal bleeding leading to the diagnosis of recurrence of a previously treated colorectal adenocarcinoma. He responded to initial therapy with systemic corticoids and anti-inhibitory coagulant complex which includes activated VII Factor [FEIBA].

Presentamos el caso de un varón de 86 años con un hematoma espontáneo en el músculo ilíaco izquierdo y diagnóstico previo de cáncer de colon en 1998 (estadio pT3N0M0), tratado quirúrgicamente mediante colectomía transversal, considerado en remisión completa. Tras realización de estudios complementarios se demostró la presencia de autoanticuerpos inhibidores del Factor VIII que confirmaron el diagnóstico de hemofilia adquirida. Durante el ingreso el paciente presentó un sangrado digestivo bajo que conllevó al descubrimiento de recidiva del adenocarcinoma colorrectal tratado previamente. Respondió de forma favorable a la terapia inicial con corticoides sistémicos y el complejo coagulante anti inhibidor que incluye el Factor VII activado [FEIBA].

[Interest of ambulatory management of patients with chronic heart failure by protocolized follow-up and therapeutic education: results of the USICAR experiment]. / Intérêt de la prise en charge ambulatoire des patients insuffisants cardiaques chroniques par suivi protocolisé et éducation thérapeutique : résultats de l'expérimentation USICAR.

OBJECTIVE: The objective of this study is to determine whether the implementation of regular and structured follow-up of patients with chronic heart failure (CHF), combined with therapeutic education, led to better management of these patients. PATIENTS AND METHOD: This was a monocentric, retrospective study on a cohort of patients with a proven CHF, followed in the Mulhouse region (France), between January 2016 and December 2017, by the Unit for Monitoring Heart Failure Patients (USICAR). These patients benefited from a regular protocolized follow-up and a therapeutic education program for a period of 2 years. The main criterion of this study was: the number of days of hospitalization for HF per year and per patient. The secondary endpoints were: the number of days of hospitalization for cardiac causes other than HF and the number of hospital stays for HF per patient. These criteria were collected over the one-year period before inclusion, at one-year-follow-up, and at two-years-follow-up. RESULTS: 159 patients with a mean age of 72.9 years were included in this study. They all had a CHF, mainly stage I-II NYHA (88.7%), of predominantly ischemic origin (50.9%), with altered left ventricular ejection fraction in 69.2% of cases. The primary endpoint averaged 8.33 days (6.84-10.13) in the year prior to inclusion, 2.6 days (1.51-4.47) in the first year of follow-up, and 2.82 days (1.30-6.11) (p <0.01 for both comparisons). The mean number of days of hospitalization for other cardiac causes other than HF to patient numbers was: 1.73 days (1.16-2.6), 1.81 days (1.04-3.16), and 1.32 days (0.57-3.08) (p = ns). The percentage of hospitalization for HF for each patient was: 69.5% (60.2-77.4) before inclusion, 16.2% (10-25.2) during the first year of follow-up and 19.3% (11-31.8) during the second (p < 0.001 for both comparisons). CONCLUSION: This study demonstrates the value of a protocolized follow-up associated with a therapeutic education program to improve the management of ambulatory CHF patients, particularly for moderate CHF.

Measles-induced Acute Disseminated Encephalomyelitis in a Non-vaccinated Patient.

We reported a case of measles-induced acute disseminated encephalomyelitis (ADEM) in a 40-year-old immunocompetent adult. The patient presented a week after the development of respiratory symptoms and a cutaneous rash, and was admitted to hospital for altered mental status. Blood tests showed hyperleukocytosis, thrombopenia and cytolysis. A lumbar puncture was consistent with acute meningitis and the patient was initially treated with antiviral and wide broad-spectrum antibiotics. Serology and PCR for measles came back positive. LEARNING POINTS: Acute disseminated encephalomyelitis (ADEM) associated with measles infection is not uncommon.Clinical suspicion must be based on the presence of measles infection or recent vaccination.A normal CT scan does not rule out the disease but magnetic resonance imaging (MRI) is not required to confirm the diagnosis.

Myxedema coma: four patients diagnosed at the Internal Medicine Department of the Dr. Negrin University Hospital in Spain.

Myxedema coma is a rare complication of hypothyroidism. Clinical examination may reveal hypotension, bradycardia, and hypothermia. Laboratory tests may show hyponatremia, lipid disorders, and elevations of creatine kinase, liver, and cardiac enzymes. We describe four cases diagnosed in our hospital during the period 1999-2017. The patients were related to amiodarone treatment, noncompliance with hormone replacement therapy, or a new diagnosis of hypothyroidism. Intravenous hormone replacement therapy was effective in three of the cases and one died. The outcome of this disease may be fatal as seen in one of our cases.

Abrupt Severe Chest Pain and Vomiting: Remember to Think of a Ruptured Oesophagus (Boerhaave Syndrome).

Boerhaave syndrome or spontaneous rupture of the oesophagus is a severe condition commonly misdiagnosed or unrecognized. Prognosis is poor even if the diagnosis is made promptly. We describe a case of Boerhaave syndrome diagnosed after the development of pneumomediastinum and cardiac arrest. Unfortunately, the patient died 48 hours after admission to the Intensive Care Unit. This entity requires a multidisciplinary management approach which may include conservative, surgical or endoscopic procedures. LEARNING POINTS: Boerhaave syndrome is a diagnostic and therapeutic challenge, and rapid diagnosis and management are crucial.A thoraco-abdominal CT scan with oesophageal opacification is the gold standard investigation.A multidisciplinary and individualized approach is needed in the management of this condition.

Hipervitaminosis B12: Nuestra experiencia y una revisión / Hypervitaminosis B12. Our experience and a review

Los altos niveles de vitamina B12 o cobalamina, también denominado hipervitaminosis B12 es una anormalidad analítica frecuentemente subestimada. De acuerdo con la literatura algunas de las entidades relacionadas con este hallazgo son las neoplasias sólidas (primarias o metastásicas) y las enfermedades hematológicas agudas o crónicas. Otras causas incluyen la afección hepática, la gammapatía monoclonal de significación indeterminada, la insuficiencia renal y, con menor frecuencia, un exceso de consumo de vitamina B12, enfermedades inflamatorias o autoinmunes y los trastornos hematológicos transitorios (neutrofilia y eosinofilia secundaria). Este artículo informa sobre causas de hipervitaminosis B12, nuestra experiencia y hace una revisión de la literatura.

High serum levels of vitamin B12 or cobalamin, also called hypervitaminemia B12, is a frequently underestimated biological abnormality. According to the literature, some of the entities related to this finding are solid neoplasia (primary or metastatic) and acute or chronic hematological diseases. Other causes include liver disorders, monoclonal gammapathy of undetermined significance, renal failure and, less frequently, excess of vitamin B12 intake, inflammatory or autoimmune diseases, and transient hematological disorders (neutrophilia and secondary eosinophilia). This article reports on causes of hypervitaminosis B12, our experience and a review of the literature.

State of Art of Idiosyncratic Drug-Induced Neutropenia or Agranulocytosis, with a Focus on Biotherapies.

INTRODUCTION: Idiosyncratic drug-induced neutropenia and agranulocytosis is seldom discussed in the literature, especially for new drugs such as biotherapies outside the context of oncology. In the present paper, we report and discuss the clinical data and management of this relatively rare disorder, with a focus on biotherapies used in autoimmune and auto-inflammatory diseases. MATERIALS AND METHODS: A review of the literature was carried out using the PubMed database of the US National Library of Medicine. We searched for articles published between January 2010 and May 2019 using the following key words or associations: "drug-induced neutropenia", "drug-induced agranulocytosis", and "idiosyncratic agranulocytosis". We included specific searches on several biotherapies used outside the context of oncology, including: tumor necrosis factor (TNF)-alpha inhibitors, anti-CD20 agents, anti-C52 agents, interleukin (IL) 6 inhibitors, IL 1 inhibitors, and B-cell activating factor inhibitor. RESULTS: Idiosyncratic neutropenia remains a potentially serious adverse event due to the frequency of severe sepsis with severe deep tissue infections (e.g., pneumonia), septicemia, and septic shock in approximately two-thirds of all hospitalized patients with grade 3 or 4 neutropenia (neutrophil count (NC) ≤ 0.5 × 109/L and ≤ 0.1 × 109/L, respectively). Over the last 20 years, several drugs have been strongly associated with the occurrence of idiosyncratic neutropenia, including antithyroid drugs, ticlopidine, clozapine, sulfasalazine, antibiotics such as trimethoprim-sulfamethoxazole, and deferiprone. Transient grade 1-2 neutropenia (absolute blood NC between 1.5 and 0.5 × 109/L) related to biotherapy is relatively common with these drugs. An approximate 10% prevalence of such neutropenia has been reported with several of these biotherapies (e.g., TNF-alpha inhibitors, IL6 inhibitors, and anti-CD52 agents). Grade 3-4 neutropenia or agranulocytosis and clinical manifestations related to sepsis are less common, with only a few case reports to date for most biotherapies. Special mention should be made of late onset and potentially severe neutropenia, especially following anti-CD52 agent therapy. During drug therapy, several prognostic factors have been identified that may be helpful when identifying 'susceptible' patients. Older age (>65 years), septicemia or shock, renal failure, and a neutrophil count ≤0.1 × 109/L have been identified as poor prognostic factors. Idiosyncratic neutropenia should be managed depending on clinical severity, with permanent/transient discontinuation or a lower dose of the drug, switching from one drug to another of the same or another class, broad-spectrum antibiotics in cases of sepsis, and hematopoietic growth factors (particularly G-CSF). CONCLUSION: Significant progress has been made in recent years in the field of idiosyncratic drug-induced neutropenia, leading to an improvement in their prognosis (currently, mortality rate between 5 and 10%). Clinicians must continue their efforts to improve their knowledge of these adverse events with new drugs as biotherapies.

A Rare Case of Adult Acute Disseminated Encephalomyelitis Associated with Primary Epstein-Barr Virus Infection.

We report a rare case of acute disseminated encephalomyelitis (ADEM) secondary to a primary Epstein-Barr (EBV) infection, in a 22-year-old male. Symptomatic infectious mononucleosis and ADEM are both quite uncommon conditions in this age group. LEARNING POINTS: ADEM is a very rare CNS disorder; it is more common in childhood but can also be described in adults.The clinical evaluation must focus on the presence of recent signs of infection or vaccination.A normal cerebral scan does not rule out the disease and MRI must be performed to confirm the diagnoses.

Rare Case of Ischaemic Stroke Following Cervical Transforaminal Injection.

We report a case of brainstem infarction following a C5-C6 cervical transforaminal injection, a rare and serious neurological complication of this procedure. Cervical transforaminal steroid injection is a common therapy for patients with persistent cervical radiculopathy not relieved by conservative treatment, and is effective in 65-70% of cases. Unfortunately, this procedure may lead to serious complications such as neurological damage. These complications are rare but potentially fatal, as reported in our case. Complications could be due to three mechanisms: the technique itself, the cervical vascular anatomy and the properties of the product (corticoids). The neurological complications can be diagnosed through brain MRI. This case report focuses on the importance of a risk/benefit evaluation when performing this medical procedure. LEARNING POINTS: Physicians should be better informed about severe complications following cervical transforaminal epidural steroid injections and their strict indications.Severe adverse events are rare but they can be disastrous.Non-particulate corticosteroid should be used.

Síndrome del cascanueces / Nutcracker syndrome

El síndrome del cascanueces es una anomalía vascular en la que se comprime la vena renal izquierda a su paso entre la unión de la aorta y la arteria mesentérica superior, debido a un ángulo muy cerrado entre ambas arterias. Clínicamente puede presentarse como hematuria macro o microscópica, dolor episódico en flanco izquierdo, dolor pélvico, várices gonadales o simplemente cursar de forma asintomática. Presentamos dos casos clínicos, uno de ellos con dolor abdominal tipo cólico nefrítico y hematuria macroscópica y otro estudiado por hipertensión pero con antecedentes de embolización de la vena renal izquierda por varicocele izquierdo. Discutimos los aspectos clínicos, diagnósticos y terapéuticos de este síndrome.

Nutcracker syndrome is a vascular anomaly consisting in the compression of the left renal vein between the superior mesenteric artery and the aorta. Clinical features in nutcracker syndrome include pelvic pain, flank pain, haematuria, gonadal varices or simply asymptomatic. We are presenting two cases, one of them with macroscopic haematuria and flank pain and the other was studied for hypertension but with previous antecedents of left renal vein embolization in the setting of varicocele. We discuss the clinical presentation as well as diagnostic and therapeutic aspects related to this syndrome.

Hypocalcemia Revealing an Enteropathy-Associated T-cell Lymphoma.

BACKGROUND: Enteropathy-associated T-cell lymphoma (EATL) is a rare and aggressive type of extranodal T-cell lymphoma (TCL) arising in the gastrointestinal (GI) tract and represents 5-8% of all T-cell non-Hodgkin lymphomas (NHL) and 10-25% of primary intestinal lymphomas. CASE PRESENTATION: We reported a 78-year-old woman presenting with severe hypocalcemia. Investigations confirmed vitamin D and iron deficiency as well as hypoalbuminemia. Celiac disease was suspected and confirmed, but despite intravenous calcium and magnesium supplementation and a gluten-free diet, normal electrolyte levels were never reached. Intestinal perforation was the clue to the diagnosis of enteropathy-associated T-cell lymphoma (EATL). CONCLUSION: Hypocalcemia can result from multiple conditions. In patients not responding to adequate supplementation, further investigations should be performed to diagnose the underlying condition.

[Predictive factors of early readmission and mortality in patients with heart failure hospitalized in the Department of Internal Medicine of the San Carlos University Hospital, Spain]. / Facteurs de prédiction de réadmission précoce et mortalité dans l'insuffisance cardiaque dans le Service de Médecine Interne de l'Hôpital Universitaire San Carlos, Espagne.

INTRODUCTION: Heart failure (HF) is a health problem in Spain where the prevalence rate for this disease is correlated with aging. Heart failure-related mortality and hospital readmissions are high. The purpose of this study was to evaluate the clinical features of patients with HF hospitalized in the Department of Internal Medicine as well as factors associated with readmission and intra-hospital mortality. METHODS: We conducted a cross-sectional, descriptive, and retrospective study based on the review of the clinical records of patients with primary diagnosis of HF in the Basic Minimum Set of Data (BMSD, Conjunto Mínimo Básico de Datos),who were discharged from the Department of Internal Medicine of the San Carlos Clinical Hospital (HCSC) in 2014. RESULTS: The study involved 199 patients, with an average age of 82.7 years (61.8% were females); 85% of them had left ventricular ejection fraction (LVEF) > 40%, with an average pro-BNP of 9.101,3 pg/ml and 64.3% had ongoing atrial fibrillation. Thirty point two percent of patients were readmitted within 30 days, with an average rate of readmission/year of 1.45 (±0.86). Twenty five percent of patients died during the follow-up period in hospital. Among factors associated with intra-hospital mortality, older age was an associated variable (OR 1,050)(1,002-1,101) (p = 0.04). The most important factors associated with early readmission were polypharmacy (p = 0.024) as well as pluripathology based on Ollero criteria 4,974 (1,396-17,730) (p = 0.024). Patients hospitalized for HF in our Department are elderly patients treated with polymedication. CONCLUSION: Patients hospitalized for cardiac insufficiency are older and are characterized by pluripathology and polypharmacy. Short-term prognosis is associated with high rates of readmission and mortality in hospitalmainly for patients suffering from kidney disease and/or neurological disorders.

Cognitive Decline: Not Always Alzheimer's Disease.

A 66-year-old woman presented with slowly progressive cognitive decline. MRI showed bi-frontotemporal atrophy, but the 14.3.3 protein was not present in cerebrospinal fluid. Electroencephalogram was irrelevant. Interview with relatives led doctors to search for a genetic cause and confirmed the diagnosis of Gerstmann-Sträussler-Scheinker syndrome.

[Acute coronary syndrome in a young female patient: findings beyond coronary lesions]. / Syndrome coronarien aigu chez une jeune patiente: au-delà des lésions coronaires.

We here report the case of a 47-year old female patient with acute coronary syndrome associated with possible arterial embolism of the right lower limb. During examination we detected G201210A Mutation of the Prothrombin Gene associated with lupus anticoagulant factor.

Pathological changes of renal biopsy in Sjögren Syndrome.

We are presenting the case of a 53-year-old woman with a history of Sjögren syndrome and a secondary antiphospholipid syndrome admitted at the Nephrology department for the evaluation of renal failure. The patient was initially diagnosed with tubulointerstitial nephritis and subsequently a membranoproliferative type I glomerulonephritis, secondary to cryoglobulins during the course of the disease. Repeated renal biopsies were required to confirm the diagnosis.

Women with atrial fibrillation and type 2 diabetes have a higher incidence of hospitalization and undergo ablation or pacemaker implantation less frequently than men.

BACKGROUND: We reviewed trends from 2004 to 2013 in the incidence and outcomes for atrial fibrillation (AF) in Spanish patients with type 2 diabetes mellitus (T2DM) comparing women and men. METHODS: We used national hospital discharge data including all T2DM patients discharged from the hospital after AF. Patients with AF in the primary diagnosis field were selected. Discharges were grouped by sex. Incidence was calculated overall and stratified by sex. We analyzed diagnostic and therapeutic procedures, patient comorbidities, CHA2DS2-VASc score, length of hospital stay, readmission rates and in-hospital mortality (IHM). RESULTS: We identified a total of 214,457 admissions for AF. Patients with T2DM accounted for 21.1% (19,505 men and 25,954 women). Women with T2DM had a significantly higher incidence of AF compared to men over the study period (IRR 1.33;95%CI 1.31-1.35). Women were significantly older (77.24±8.69years) than men (72.62±10.28years), had higher prevalences of obesity and hypertension, and higher CHA2DS2-VASc score. Women less frequently underwent ablation (3.21% vs. 1.54%; p<0.001) and received an implanted pacemaker (14.3% vs. 8.16%; p<0.001) than men. Crude IHM was 2.81% for women and 2.48% for men (p=0.030). Sex was not associated with a higher IHM after multivariable adjustment. CONCLUSIONS: Our study demonstrates an increase in hospitalization for AF in diabetic women. Women were older, had a higher comorbidity index and had CHAD2DS2-VASc score than men. Women with AF and T2DM undergo ablation or pacemaker implantation less frequently than their male counterparts. After multivariable adjustment sex did not predict mortality during admissions for AF.

Acute Q Fever Presenting with Multi-Organ Failure: Re-Evaluation of the Initial Diagnosis.

We present the case of a 48-year-old man admitted to the critical care unit with atrial fibrillation, and acute heart and kidney failure accompanied by coagulopathy and an abnormal liver test. Initially diagnosed as a non-ST elevation myocardial infarction, re-evaluation of the case led to the consideration of severe sepsis. Q fever and leptospirosis were the most probable causes and empiric treatment was initiated. A complete recovery was achieved following treatment. LEARNING POINTS: Epidemiological antecedents of interest must always be collected in the clinical record.Acute Q fever infection may be mild or present with multiple organ damage.Empiric treatment with doxycycline must be started when Q fever is suspected.

Prasugrel and Acquired Thrombotic Thrombocytopenic Purpura Associated with ADAMTS13 Activity Deficiency.

We report a case of a 64-year-old man who, 44 days after starting treatment with prasugrel, presented with severe thrombocytopenia, anaemia, renal failure, and severe ADAMTS13 activity deficiency, along with a high titer of autoantibodies to this protease. LEARNING POINTS: Drug-induced TTP is a rare condition and difficult to diagnose.Decreased activity of ADAMTS13, unusual in drug-induced TTP, was present in this case.